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74. Electrophysiological and clinical findings in patients with epilepsy and hippocampal malrotation
Society Proceedings / Clinical Neurophysiology 119 (2008) e99–e164
71. Polysomnographic characterization in the SCA2 mutation—L.C. Vela´zquez Pe´rez, R. Rodrı´guez Labrada, L. Tuin, G. Sa´nchez Cruz, M.L. Galicia Polo, R. Haro Valencia, N. Canales Ochoa, R. Aguilera, F. Wirjatijasa, G. Auburger (Cuba) The aim of the present study was to characterize the sleep pathology in SCA2 mutation by polysomnographic studies. The authors also sought to check whether this disorder appeared in preclinical stages of the disease and to identify therapeutic targets for treatment strategies. Thirty six SCA2 patients, presymptomatics and sex-and age-matched healthy controls were studied by polysomnography and sleep interviews. The polyglutamine expansion size and clinical scores were obtained in SCA2 mutation carriers and SCA2 patients groups, respectively. Presymptomatics and SCA2 patients reported good subjective sleep quality and no incidents of REM behavior disorders (RBD). However, REM sleep was abnormal in 60% of SCA2 patients and presymptomatics. The anomalies of REM sleep were time reduction, insufficient muscle atonia and REM density decrease. Abnormal motor control during sleep with periodic legs movements (PLMs) was found. SCA2 patients and presymptomatics showed sleep efficiency reduction and increase of arousal index and slow wave sleep. The early and progressive REM sleep reduction can be associated with the pons, nigrostriatal and thalamic degeneration. REM sleep without atonia may be interpreted as subclinical RBD and suggest neurodegenerative lesions in subceruleus region. PLMs may be related to a dysfunction of dopaminergic pathways, therefore it may be treated with dopaminergic therapy doi:10.1016/j.clinph.2008.04.087
72. Saccadic movements in SCA2: From disorders to electrophysiological biomarkers for clinicogenetic researches— R. Rodrı´guez Labrada, L.C. Vela´zquez Pe´rez, C. Seifried, U. Ziemann, G. Sa´nchez Cruz, N. Canales Ochoa, Y. Medrano, G. Auburger (Cuba) The author’s purpose was to characterize the saccadic abnormalities related to SCA2 mutation by electronystagmography. We also aimed to evaluate their correlation with disease duration, ataxia score and polyglutamine expansion. Saccade velocity, latency and deviation were evaluated in 82 SCA2 patients, 53 asymptomatic carriers and 82 healthy controls studied by electronystagmography. SCA2 patients showed a significant reduction of maximal saccade velocity (MSV), prolonged latency and hypometric saccades to predictable amplitude. Significant saccadic slowing, but less severe than patients, was observed in presymptomatics. MSV was negatively correlated with the polyglutamine expansion in both groups. In the group of SCA2 patients, saccadic abnormalities were significantly accented along time. The saccadic slowing and hypometria showed a larger magnitude of progression than latency prolongation compared with controls. Saccade velocity is an important electrophysiological research tool for the study of genetic determinants of SCA2. The progression patterns of saccade slowing and hypometria appear to be valuable biomarkers that reflect the severity of neurodegenerative
e117
process in the brainstem and cerebellum along time in SCA2 patients. doi:10.1016/j.clinph.2008.04.088
73. Sympathetic skin response and heart rate variability in patients with spinocerebellar Ataxia Type 2—G. Sa´nchez Cruz, L.C. Vela´zquez Pe´rez, J. Montes Brown, N. Canales Ochoa, R. Rodrı´guez Labrada (Cuba) The aim of the present study was to evaluate the functional state of the autonomous nervous system in patients with diagnosis of Spinocerebellar Ataxia II using electrophysiological methods. The sympathetic skin response and the heart rate variability were studied in 84 and 103 patients, respectively. These groups were compared to age-sex matched healthy control subjects. Molecular genetic determination, clinical ataxia and clinical autonomic scores were also studied. The sympathetic skin response latencies in patients were prolonged compared to controls (p = .019) and amplitudes in patients were smaller compared to controls (mean palm, p = .001 and mean sole, p = .003). The patients showed significant lower heart rate variability indexes in the time domain, and significant differences in the frequency domain, overall p < .005. In addition, we found a significant correlation between disease’s duration and sympathetic skin response variables (p < .005). Some of the HRV variables correlated with the time evolution, and the CAG repeat (SD and MSSD breathing at rest, E/I ratio, LF/HF ratio, and others (p < .005). The SCA2 patients showed a dysfunction of the autonomous nervous system with the sympathetic and parasympathetic branch affected. doi:10.1016/j.clinph.2008.04.089
74. Electrophysiological and clinical findings in patients with epilepsy and hippocampal malrotation—G. Kuester, J.C. Casar, J. Godoy, J. Santo´n, T. Mesa (Chile) Our aim was to describe electrophysiological and clinical findings in epilepsy patients with hippocampal malrotation (HM). We retrospectively reviewed all consecutive patients studied with prolonged video-EEG monitoring, from January 2004 to December 2006. Patients with MRI showing medially placed/round/vertically oriented hippocampus, and collateral sulcus deep, verticalized or protruding into empty choroid fissure were selected. We analyzed demography, pre- and perinatal history, history of febrile seizures, age of epilepsy onset, neurological/mental state, and type of seizures, AED response, and evidence of other lesions such as malformations of cortical development or hippocampal sclerosis. We correlated basal/interictal/ictal EEG findings with MRI features. Seven patients were included, six had left HM. Mean age: 12.7 years (range: 20 mo.–30 years), four male. Mean age at epilepsy onset: 5.8 years (range: 3 mo.–22 years). Four patients had familiar history of epilepsy, three had perinatal pathology, and two had febrile seizures. Five patients had pharmacoresistant focal epilepsy, one SMEI, and one had non-refractory focal epilepsy (with right HM). Neurological examination was abnormal in six. Only one patient had unifocal interictal/ictal epileptiform activity and showed good correlation with HM. In
e118
Society Proceedings / Clinical Neurophysiology 119 (2008) e99–e164
this small series of epilepsy patients with HM, left hippocampus was more frequently affected. Interictal/ictal epileptiform activity was rarely unifocal and good relationship between HM and EEG abnormalities was rare. doi:10.1016/j.clinph.2008.04.090
75. The electroencephalogram and the neurodyinamic esthesiometry in healthy adults of different ages—M. Cisneros Cue´, J.A. Cıas, C.N. Pe´rez Lache, R.A. Riba (Cuba) The neurodyinamic esthesiometric method (NEM) of the discriminative tactile perception, evaluates the force and mobility of the cortical cerebral processes of cutaneous-kinesthesic analyzer. The objectives of this work were to use the EEG like an indicator of the utility of NEM in the detection of functional cerebral variations provoked by aging and to support the clinical approach for the selection of normal adults of different ages. Sixty healthy adults, without personal or familiar antecedents of neurological, psychiatric or chronic disease with negative repercussion on the CNS, without cognitive deficit and a normal neurological exam were selected. They were distributed in S1/20–39, S2/40–59 and S3/60–79 year’s old subgroups. A NEM was carried out in all of them and the EEG was recorded in 80% of the sample. The alpha absolute power and its mean frequency diminished with age. The alpha relative power was smaller in S3 than in S1. The beta relative power was scarce in S1 and normal in S2 and S3. Normal NEM: S1-100%, S2-75% and S3-70%, the parameters indicate a reduction of values with age. The EEG reaffirms the utility of NEM to evaluate the functional cerebral status, as well as it supports the validity of the clinical approach used for the selection of healthy adults. doi:10.1016/j.clinph.2008.04.091
Analog Scale dropped from 9 before treatment to 1 after treatment. The improvement in both subjective pain and sleep supports the general association between pain and poor slow-wave sleep. If replicated in a larger population, sodium oxybate may be a treatment option for fibromyalgia that differs from conventional analgesics. The results also suggest the possibility of QEEG during polysomnography as a surrogate marker of treatment in fibromyalgia. doi:10.1016/j.clinph.2008.04.092
77. Electroencephalographic activity during visual sustained attention task in primary insomnia—M. Corsi Cabrera, A. Pe´rez Ortiz, J.I. Sa´nchez Romero, P. Figueredo Rodrı´guez, ´ o Portilla (Mexico) M.A. Guevara, Y. Del Rı The goal of this study was to determine the differences in the electrophysiological pattern during the execution of a visual sustained attentional task between subjects with primary insomnia and a control group. Comparison was made between 8 patients with primary insomnia and 8 subjects without any sleep disorders. Ages ranged between 19 and 35 years and all subjects were right handed. During the visual sustained attentional task execution each subject had to press a key in response to the appearance of certain stimulus at the center of a computer screen. The entire task lasted about 10 min. Higher right intrahemispheric EEG correlation was observed in insomniacs together with theta band decrease and increases in the beta and gamma bands as compared with the control group. These last results are in close relation with higher activation in insomniacs. During the task execution before sleeping, insomniacs had higher absolute theta and alpha power in frontal (attention) and middles frontal areas (activation through the cingulus). These results suggest that insomniacs have higher coherent attention systems than controls. It would explain the diurnal consequences affecting their daily functioning. doi:10.1016/j.clinph.2008.04.093
76. Quantitative EEG during nocturnal polysomnography identifies dysfunctional sleep in fibromyalgia and measures response to treatment—V.W. Rosenfeld, S.E. Nielsen, D. Nguyen, M. Stern (USA) Fibromyalgia is a pain syndrome that is associated with dysfunctional sleep, frequent awakenings, non-refreshing sleep and alpha frequency intrusions in slow wave sleep. Because the GABA metabolite sodium oxybate increases delta sleep and reduces alpha intrusions, we assessed whether it may improve objective sleep findings and subjective pain symptoms of fibromyalgia. One patient with fibromyalgia underwent all-night polysomnogram. After 6 months of treatment with sodium oxybate, the polysomnogram was repeated. Quantitative electroencephalogram analysis determined the amount of alpha and delta activity in all sleep stages. Standard pain inventories were obtained before and after treatment. During slow wave sleep (SWS), 68 alpha events (aes) occurred before and 66 after treatment with sodium oxybate, and 2655 delta events (des) occurred before and 3701 after treatment. Events are five or more cycles in the frequency range. The DE/AEP ratio improved from 39.04 to 56.07 after treatment with sodium oxybate. Pain as measured by the Visual
78. The mismatch negativity ERP in two subtypes of children with developmental dyslexia—D.M. Herna´ndez Barros, M.C. Pe´rez Abalo, R.M. Morgades Fonte, V. Reigosa Crespo, L. Gala´n Garcı´a, E. Santos Febles (Cuba) The aim of this study was to evaluate with the mismatch negativity (MMN) in two subtypes of developmental dyslexia. Fourteen dyslexic children and seven siblings were examined. Using a words and pseudowords reading task, the dyslexic group was divided into two subtypes, phonologic or surface dyslexics, respectively. A mismatch negativity (MMN) was measured in all this subjects, using the classical paradigm of auditory discrimination of pure tones (1000 and 1200 Hz) presented sequentially with a relative probability of 0.15 y 0.85 and 600 ms of interstimulus interval. An MMN was obtained to both, control subjects and the surface deficit group. Dyslexics with phonological deficit haven’t got this component. Differences in electrophysiological patterns of these two subtypes of developmental dyslexia, contradict the idea of a unique cognitive mechanism damaged in this impairment. The highest differences in the amplitude in MMN
71. Polysomnographic characterization in the SCA2 mutation—L.C. Vela´zquez Pe´rez, R. Rodrı´guez Labrada, L. Tuin, G. Sa´nchez Cruz, M.L. Galicia Polo, R. Haro Valencia, N. Canales Ochoa, R. Aguilera, F. Wirjatijasa, G. Auburger (Cuba) The aim of the present study was to characterize the sleep pathology in SCA2 mutation by polysomnographic studies. The authors also sought to check whether this disorder appeared in preclinical stages of the disease and to identify therapeutic targets for treatment strategies. Thirty six SCA2 patients, presymptomatics and sex-and age-matched healthy controls were studied by polysomnography and sleep interviews. The polyglutamine expansion size and clinical scores were obtained in SCA2 mutation carriers and SCA2 patients groups, respectively. Presymptomatics and SCA2 patients reported good subjective sleep quality and no incidents of REM behavior disorders (RBD). However, REM sleep was abnormal in 60% of SCA2 patients and presymptomatics. The anomalies of REM sleep were time reduction, insufficient muscle atonia and REM density decrease. Abnormal motor control during sleep with periodic legs movements (PLMs) was found. SCA2 patients and presymptomatics showed sleep efficiency reduction and increase of arousal index and slow wave sleep. The early and progressive REM sleep reduction can be associated with the pons, nigrostriatal and thalamic degeneration. REM sleep without atonia may be interpreted as subclinical RBD and suggest neurodegenerative lesions in subceruleus region. PLMs may be related to a dysfunction of dopaminergic pathways, therefore it may be treated with dopaminergic therapy doi:10.1016/j.clinph.2008.04.087
72. Saccadic movements in SCA2: From disorders to electrophysiological biomarkers for clinicogenetic researches— R. Rodrı´guez Labrada, L.C. Vela´zquez Pe´rez, C. Seifried, U. Ziemann, G. Sa´nchez Cruz, N. Canales Ochoa, Y. Medrano, G. Auburger (Cuba) The author’s purpose was to characterize the saccadic abnormalities related to SCA2 mutation by electronystagmography. We also aimed to evaluate their correlation with disease duration, ataxia score and polyglutamine expansion. Saccade velocity, latency and deviation were evaluated in 82 SCA2 patients, 53 asymptomatic carriers and 82 healthy controls studied by electronystagmography. SCA2 patients showed a significant reduction of maximal saccade velocity (MSV), prolonged latency and hypometric saccades to predictable amplitude. Significant saccadic slowing, but less severe than patients, was observed in presymptomatics. MSV was negatively correlated with the polyglutamine expansion in both groups. In the group of SCA2 patients, saccadic abnormalities were significantly accented along time. The saccadic slowing and hypometria showed a larger magnitude of progression than latency prolongation compared with controls. Saccade velocity is an important electrophysiological research tool for the study of genetic determinants of SCA2. The progression patterns of saccade slowing and hypometria appear to be valuable biomarkers that reflect the severity of neurodegenerative
e117
process in the brainstem and cerebellum along time in SCA2 patients. doi:10.1016/j.clinph.2008.04.088
73. Sympathetic skin response and heart rate variability in patients with spinocerebellar Ataxia Type 2—G. Sa´nchez Cruz, L.C. Vela´zquez Pe´rez, J. Montes Brown, N. Canales Ochoa, R. Rodrı´guez Labrada (Cuba) The aim of the present study was to evaluate the functional state of the autonomous nervous system in patients with diagnosis of Spinocerebellar Ataxia II using electrophysiological methods. The sympathetic skin response and the heart rate variability were studied in 84 and 103 patients, respectively. These groups were compared to age-sex matched healthy control subjects. Molecular genetic determination, clinical ataxia and clinical autonomic scores were also studied. The sympathetic skin response latencies in patients were prolonged compared to controls (p = .019) and amplitudes in patients were smaller compared to controls (mean palm, p = .001 and mean sole, p = .003). The patients showed significant lower heart rate variability indexes in the time domain, and significant differences in the frequency domain, overall p < .005. In addition, we found a significant correlation between disease’s duration and sympathetic skin response variables (p < .005). Some of the HRV variables correlated with the time evolution, and the CAG repeat (SD and MSSD breathing at rest, E/I ratio, LF/HF ratio, and others (p < .005). The SCA2 patients showed a dysfunction of the autonomous nervous system with the sympathetic and parasympathetic branch affected. doi:10.1016/j.clinph.2008.04.089
74. Electrophysiological and clinical findings in patients with epilepsy and hippocampal malrotation—G. Kuester, J.C. Casar, J. Godoy, J. Santo´n, T. Mesa (Chile) Our aim was to describe electrophysiological and clinical findings in epilepsy patients with hippocampal malrotation (HM). We retrospectively reviewed all consecutive patients studied with prolonged video-EEG monitoring, from January 2004 to December 2006. Patients with MRI showing medially placed/round/vertically oriented hippocampus, and collateral sulcus deep, verticalized or protruding into empty choroid fissure were selected. We analyzed demography, pre- and perinatal history, history of febrile seizures, age of epilepsy onset, neurological/mental state, and type of seizures, AED response, and evidence of other lesions such as malformations of cortical development or hippocampal sclerosis. We correlated basal/interictal/ictal EEG findings with MRI features. Seven patients were included, six had left HM. Mean age: 12.7 years (range: 20 mo.–30 years), four male. Mean age at epilepsy onset: 5.8 years (range: 3 mo.–22 years). Four patients had familiar history of epilepsy, three had perinatal pathology, and two had febrile seizures. Five patients had pharmacoresistant focal epilepsy, one SMEI, and one had non-refractory focal epilepsy (with right HM). Neurological examination was abnormal in six. Only one patient had unifocal interictal/ictal epileptiform activity and showed good correlation with HM. In
e118
Society Proceedings / Clinical Neurophysiology 119 (2008) e99–e164
this small series of epilepsy patients with HM, left hippocampus was more frequently affected. Interictal/ictal epileptiform activity was rarely unifocal and good relationship between HM and EEG abnormalities was rare. doi:10.1016/j.clinph.2008.04.090
75. The electroencephalogram and the neurodyinamic esthesiometry in healthy adults of different ages—M. Cisneros Cue´, J.A. Cıas, C.N. Pe´rez Lache, R.A. Riba (Cuba) The neurodyinamic esthesiometric method (NEM) of the discriminative tactile perception, evaluates the force and mobility of the cortical cerebral processes of cutaneous-kinesthesic analyzer. The objectives of this work were to use the EEG like an indicator of the utility of NEM in the detection of functional cerebral variations provoked by aging and to support the clinical approach for the selection of normal adults of different ages. Sixty healthy adults, without personal or familiar antecedents of neurological, psychiatric or chronic disease with negative repercussion on the CNS, without cognitive deficit and a normal neurological exam were selected. They were distributed in S1/20–39, S2/40–59 and S3/60–79 year’s old subgroups. A NEM was carried out in all of them and the EEG was recorded in 80% of the sample. The alpha absolute power and its mean frequency diminished with age. The alpha relative power was smaller in S3 than in S1. The beta relative power was scarce in S1 and normal in S2 and S3. Normal NEM: S1-100%, S2-75% and S3-70%, the parameters indicate a reduction of values with age. The EEG reaffirms the utility of NEM to evaluate the functional cerebral status, as well as it supports the validity of the clinical approach used for the selection of healthy adults. doi:10.1016/j.clinph.2008.04.091
Analog Scale dropped from 9 before treatment to 1 after treatment. The improvement in both subjective pain and sleep supports the general association between pain and poor slow-wave sleep. If replicated in a larger population, sodium oxybate may be a treatment option for fibromyalgia that differs from conventional analgesics. The results also suggest the possibility of QEEG during polysomnography as a surrogate marker of treatment in fibromyalgia. doi:10.1016/j.clinph.2008.04.092
77. Electroencephalographic activity during visual sustained attention task in primary insomnia—M. Corsi Cabrera, A. Pe´rez Ortiz, J.I. Sa´nchez Romero, P. Figueredo Rodrı´guez, ´ o Portilla (Mexico) M.A. Guevara, Y. Del Rı The goal of this study was to determine the differences in the electrophysiological pattern during the execution of a visual sustained attentional task between subjects with primary insomnia and a control group. Comparison was made between 8 patients with primary insomnia and 8 subjects without any sleep disorders. Ages ranged between 19 and 35 years and all subjects were right handed. During the visual sustained attentional task execution each subject had to press a key in response to the appearance of certain stimulus at the center of a computer screen. The entire task lasted about 10 min. Higher right intrahemispheric EEG correlation was observed in insomniacs together with theta band decrease and increases in the beta and gamma bands as compared with the control group. These last results are in close relation with higher activation in insomniacs. During the task execution before sleeping, insomniacs had higher absolute theta and alpha power in frontal (attention) and middles frontal areas (activation through the cingulus). These results suggest that insomniacs have higher coherent attention systems than controls. It would explain the diurnal consequences affecting their daily functioning. doi:10.1016/j.clinph.2008.04.093
76. Quantitative EEG during nocturnal polysomnography identifies dysfunctional sleep in fibromyalgia and measures response to treatment—V.W. Rosenfeld, S.E. Nielsen, D. Nguyen, M. Stern (USA) Fibromyalgia is a pain syndrome that is associated with dysfunctional sleep, frequent awakenings, non-refreshing sleep and alpha frequency intrusions in slow wave sleep. Because the GABA metabolite sodium oxybate increases delta sleep and reduces alpha intrusions, we assessed whether it may improve objective sleep findings and subjective pain symptoms of fibromyalgia. One patient with fibromyalgia underwent all-night polysomnogram. After 6 months of treatment with sodium oxybate, the polysomnogram was repeated. Quantitative electroencephalogram analysis determined the amount of alpha and delta activity in all sleep stages. Standard pain inventories were obtained before and after treatment. During slow wave sleep (SWS), 68 alpha events (aes) occurred before and 66 after treatment with sodium oxybate, and 2655 delta events (des) occurred before and 3701 after treatment. Events are five or more cycles in the frequency range. The DE/AEP ratio improved from 39.04 to 56.07 after treatment with sodium oxybate. Pain as measured by the Visual
78. The mismatch negativity ERP in two subtypes of children with developmental dyslexia—D.M. Herna´ndez Barros, M.C. Pe´rez Abalo, R.M. Morgades Fonte, V. Reigosa Crespo, L. Gala´n Garcı´a, E. Santos Febles (Cuba) The aim of this study was to evaluate with the mismatch negativity (MMN) in two subtypes of developmental dyslexia. Fourteen dyslexic children and seven siblings were examined. Using a words and pseudowords reading task, the dyslexic group was divided into two subtypes, phonologic or surface dyslexics, respectively. A mismatch negativity (MMN) was measured in all this subjects, using the classical paradigm of auditory discrimination of pure tones (1000 and 1200 Hz) presented sequentially with a relative probability of 0.15 y 0.85 and 600 ms of interstimulus interval. An MMN was obtained to both, control subjects and the surface deficit group. Dyslexics with phonological deficit haven’t got this component. Differences in electrophysiological patterns of these two subtypes of developmental dyslexia, contradict the idea of a unique cognitive mechanism damaged in this impairment. The highest differences in the amplitude in MMN